If untreated, the vast majority of patients can die within a year. Current treatments improve prognosis, but affected patients remain at a substantial...
If untreated, the vast majority of patients can die within a year. Current treatments improve prognosis, but affected patients remain at a substantially higher risk of death and adverse outcomes.
Since the discovery of anti-neutrophil cytoplasmic antibodies (ANCA) in 1982, enormous progress has been made in the understanding of the associated diseases and their treatment. From a diagnostic viewpoint, the elucidation of proteinase 3 (PR3) and myeloperoxidase (MPO) as the relevant antigenic specificities of ANCA in the ANCA-associated vasculitis (AAV) has strongly improved early diagnosis and treatment of these diseases.
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Treatment of AAV follows type- and activity-adapted regimens according to evidence from controlled trials which have been summarized in the EULAR (European League against Rheumatism) recommendations published in 2009.
In general, a period of remission induction using highly-potent immunosuppression (e.g. with cyclophosphamide, Cyc plus glucocorticoids, GC) for 3–4 months is followed by maintenance therapy (e.g. azathioprine plus low-dose GC) which is supposed to be kept for at least 1.5–2 years. Induction therapy with a combination of high-dose steroids and cyclophosphamide has been the standard therapy for over 30 years and greatly improves survival among patients with AAV. Current research is focused on improving efficacy and reducing side effects of the medications used to induce remission.
For EGPA, the current approach to disease management is primarily based on reduction of active inflammation and suppression of the immune response through the use of corticosteroids and concomitant immunosuppressive therapy (e.g., methotrexate, azathioprine, mycophenolate mofetil) and/or cytotoxic agents (e.g. cyclophosphamide). Although the use of these treatments can be effective for establishing remission, patients remain vulnerable to either the complications of the long-term use of these therapies and to the risk of relapse, particularly if the dose of corticosteroid is reduced.
Original Source:- Vasculitis Market Research Report
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